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Abstract

Keratomalaeia is a necrotic corneal process, appearing as a localized or generalized area of softening of the tissue and resulting in descemetocele or corneal perforation. General malnutrition, especially hypoproteinemia and Vitamin A deficiency, are primarily responsible for the corneal affection. Secondary infection may follow.

The condition is commonly observed in areas with deficient food supplies, although it can be observed sporadically in well-developed countries. Two such sporadic cases will be reported: one patient with malnutrition due to parents' negligence, and one patient who had had a severe, long-standing gastroenteritis.

Case Reports

Case 1: A 1 1/2-year-old Bedouin boy was referred to the Pediatrics Department of the Poriah Hospital, Tiberias, in November, 1969, because of malnutrition, diarrhea, bronchopneumonia, and ulcerations of the corneas in both eyes.

The mother of the child, who was mentally disturbed, had been feeding the child mainly on biscuits and tea for the last eight months. The child could not sit or stand by himself and weighed only 7.400 Kg. Generalized edema was present, mainly in the lower extremities which also presented superficial ulcers. The child vomited and had a mucous diarrhea. A cheilitis was present.

A severe keratomalaeia was present in the right eye. In the center of the annular ulcer an iris prolapse was observed. The ulcer was partially covered with a whitish exudate (Fig. 1). The left cornea also presented an ulceration, and a prolapse of the iris could be observed at 7 o'clock near the limbus. Cultures obtained from the conjunctival sac demonstrated Staphylococus aureus, sensitive to Erythromycin.

Laboratory examinations demonstrated anemia and hypoproteinemia. Vitamin A serum level was 10 U per cent. Ascaris lumbricoides was found in the feces. Treatment consisted of blood transfusions and multivitaminic supply along with intravenous Erythromycin 100 mg. 4 times a day. Topically, atropine 1% and Erythromycin 1% ointment were administered 4 times a day. The general condition improved considerably within eight days, but the corneal condition was not better. It was decided to perform a perforating corneal graft in the right eye, and in the left eye a lamellar graft, covering the smaller corneal ulceration. A 7 mm perforating corneal graft was performed in the right eye, and the necrotic corneal tissue, the adherent iris, and the complicated cataract removed. Vitreous was removed with a cellulose sponge to avoid its contact with the graft. In the left cornea a lamellar graft was performed, after the ulcerated area was properly cleaned, leaving the small area of adherent iris in place. The perforating graft in the right eye became opaque and vascularized soon after the intervention, but the eye quieted down and the ulcerations did not recur. The left eye became quiet and the' cornea remained transparent opposite the pupillary area.

In order to prevent a perforation, a tectonic fuUthickness graft covering the whole cornea was sutured to the sclera around the limbus (Fig. 8). One week after the operation, some of the sutures gave way and it was necessary to resuture the graft. Thereon a scarring process took place and three months after surgery, the eye was quiet. A residual leucoma cornea was observed (Fig. 9). A perforating keratoplasty will be attempted in the near future. The infant is otherwise healthy.

Comment

Keratomalacia is common in underdeveloped countries, where scarcity of food is associated with little knowledge of the basic dietetic necessities of children, but very rarely seen elsewhere. The main causes are probably vitamin A and protein deficiency, although superimposed or triggering infections have been found in many cases,1 especially in the more severe forms of malnutrition.

Keratomalaeia is a necrotic corneal process, appearing as a localized or generalized area of softening of the tissue and resulting in descemetocele or corneal perforation. General malnutrition, especially hypoproteinemia and Vitamin A deficiency, are primarily responsible for the corneal affection. Secondary infection may follow.

The condition is commonly observed in areas with deficient food supplies, although it can be observed sporadically in well-developed countries. Two such sporadic cases will be reported: one patient with malnutrition due to parents' negligence, and one patient who had had a severe, long-standing gastroenteritis.

Case Reports

Case 1: A 1 1/2-year-old Bedouin boy was referred to the Pediatrics Department of the Poriah Hospital, Tiberias, in November, 1969, because of malnutrition, diarrhea, bronchopneumonia, and ulcerations of the corneas in both eyes.

The mother of the child, who was mentally disturbed, had been feeding the child mainly on biscuits and tea for the last eight months. The child could not sit or stand by himself and weighed only 7.400 Kg. Generalized edema was present, mainly in the lower extremities which also presented superficial ulcers. The child vomited and had a mucous diarrhea. A cheilitis was present.

A severe keratomalaeia was present in the right eye. In the center of the annular ulcer an iris prolapse was observed. The ulcer was partially covered with a whitish exudate (Fig. 1). The left cornea also presented an ulceration, and a prolapse of the iris could be observed at 7 o'clock near the limbus. Cultures obtained from the conjunctival sac demonstrated Staphylococus aureus, sensitive to Erythromycin.

Laboratory examinations demonstrated anemia and hypoproteinemia. Vitamin A serum level was 10 U per cent. Ascaris lumbricoides was found in the feces. Treatment consisted of blood transfusions and multivitaminic supply along with intravenous Erythromycin 100 mg. 4 times a day. Topically, atropine 1% and Erythromycin 1% ointment were administered 4 times a day. The general condition improved considerably within eight days, but the corneal condition was not better. It was decided to perform a perforating corneal graft in the right eye, and in the left eye a lamellar graft, covering the smaller corneal ulceration. A 7 mm perforating corneal graft was performed in the right eye, and the necrotic corneal tissue, the adherent iris, and the complicated cataract removed. Vitreous was removed with a cellulose sponge to avoid its contact with the graft. In the left cornea a lamellar graft was performed, after the ulcerated area was properly cleaned, leaving the small area of adherent iris in place. The perforating graft in the right eye became opaque and vascularized soon after the intervention, but the eye quieted down and the ulcerations did not recur. The left eye became quiet and the' cornea remained transparent opposite the pupillary area.

Fig. 4. Case 1. Right cornea. Epithelium is thinned and covered with keratin.

Fig. 5. Case 1. Right cornea. An area of parakeratosis is observed. Hematoxilin-cosin x55.

The histological sections of the right cornea, removed during the operation, demonstrated an ulcerated, necrotic area, well-delineated from normallooking corneal lamelle. In the necrotic area, numerous lymphocytes, polymorphonuclears, and debris of these cells were observed. New vessels were seen deep in this area (Fig. 2). In the center of the ulcer, the hyperplastic epithelium was observed, filling the area, and occasional keratinization could be seen (Fig. 3). The epithelium of the remainder of the cornea was thinned over areas and was covered by a layer of kératine (Fig. 4). In places, parakeratosis was observed (Fig. 5).

Case 2: A 5-month-old infant was admitted to the Pediatric Department because of severe diarrhea and vomiting of unknown origin during the past three months. On admission, the infant weighed 4.600 Kg, was malnourished and dehydrated, and had widespread dermatitis with ulcerations, mainly around the mucocutaneous junctions but also on the scalp, ears, and extremities. Severe cheilitis was present (Fig. 6). The right cornea presented an extensive, ring-shaped ulcer and a central descemetocele (Fig. 7). The anterior chamber could not be seen. The left cornea presented a small infiltration at 11 o'clock. Laboratory findings showed anemia, hypoproteinemia, hypocholesterolemia, and low serum levels of iron and vitamin Bi 2· The level of vitamin A could not be determined because the infant had received, previous to admission, a high dose of vitamin A intramuscularly. Blood and eye cultures revealed Pseudomonas aeruginosa.

Treatment consisted of intravenous fluids, (electrolytes, plasma, and blood) multivitamin preparations, and a combination of Gentamicin (Garamycin) and Carbeniciilin (Py open). The infant was fed on a Nutramigen formula. Her general condition ameliorated rapidly. Local treatment, consisting of subconjunctival Carbeniciilin, topical atropine 1%, and Syn thorny cetin 5% failed to improve the right cornea.

Fig. 6. Case 2. Widespread dermatitis with ulcerations on scalp, around the mouth and hands. Cheilitis is observed.

Fig. 9. Case 2. Postoperative leucoma cornea.

Fig. 7. Case 2. Rigill cornea. Extensive ulcer and descematocele.

Fig. 8. Case 2. Tectonic graft sutured in place.

In order to prevent a perforation, a tectonic fuUthickness graft covering the whole cornea was sutured to the sclera around the limbus (Fig. 8). One week after the operation, some of the sutures gave way and it was necessary to resuture the graft. Thereon a scarring process took place and three months after surgery, the eye was quiet. A residual leucoma cornea was observed (Fig. 9). A perforating keratoplasty will be attempted in the near future. The infant is otherwise healthy.

Comment

Keratomalacia is common in underdeveloped countries, where scarcity of food is associated with little knowledge of the basic dietetic necessities of children, but very rarely seen elsewhere. The main causes are probably vitamin A and protein deficiency, although superimposed or triggering infections have been found in many cases,1 especially in the more severe forms of malnutrition.

The first case presented in this paper was related to a deficient diet, and the second to chronic diarrhea and secondary food malabsorption. In both patients an infective agent was cultured from the eye. The histopathologic findings in Case 1 were typical of keratomalacia. These consisted of loss of corneal epithelium and its conversion, in places, to an epidermal type with superficial and deep keratinization, areas of parakeratosis, and a defined area of necrosis containing inflammatory cells and cellular debris.

In Case 2, the appearance of bilateral corneal ulcers when the infant was in a very poor general condition with severe hypoproteinemia and hypovitaminosis also points to a nutritional keratopathy.

Summary

Two sporadic cases of keratomalacia in Israel are described. In a 1 ½-year-old infant the bilateral keratopathy originated from a longstanding diet poor in proteins and vitamins. The more affected cornea was removed and the histopathologic findings were typical. In a 5 months-old baby, the bilateral keratomalacia followed a chronic diarrhea and secondary malabsortion. In both patients, cultures obtained from the conjunctival sacs revealed pathogenic microorganisms.

Acknowledgment: We acknowledge Dr. Shoshana Barkay of the Ophthalmologic Department and Dr. Roland Geiger of the Pediatrics Department of Poriah Hospital, Tiberias, for permitting us to publish Case 1 and for their help.